eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Coronary Artery Fistula: Differential Diagnoses & Workup
Updated: May 28, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Arteriovenous Fistulae, Pulmonary
Patent Ductus Arteriosus
Sinus of Valsalva Aneurysm
Other Problems to Be Considered
Ruptured sinus of Valsalva aneurysm
Ventricular septal defect with aortic insufficiency
Venous hums
Intrathoracic systemic fistulae
Workup
Imaging Studies
The following studies may be indicated in patients with coronary artery fistulae (CAF):
- Two-dimensional echocardiograms may reveal left atrial and left ventricular enlargement as a consequence of significant shunt flow or decreased regional or global dysfunction as a consequence of myocardial ischemia. The feeding coronary artery often appears enlarged, ectatic, and tortuous. High-volume flow may be detected by color-flow imaging at the origin or along the length of the vessel. Carefully seek the site of drainage; often, it is evident as a disturbed flow signal, most frequently within the right ventricle.
- Cardiac catheterization remains the modality of choice for defining coronary artery patterns of structure and flow. Most frequently, intracardiac pressures are normal and shunt flow is modest. Aortography (see Media file 1) or selective coronary arteriography (see Media file 2) supplies the information required to manage the condition. In addition, therapeutic embolization using occlusive coils or devices may be performed via catheterization.5
Selective left coronary artery (LCA) injection demonstrating a markedly enlarged left main (*) with normal size circumflex (CX) and left anterior descending (LAD) branches. The fistula continues across the right ventricle free wall to the atrioventricular groove where it terminates at the crux of the heart in the right atrium (straight arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
Retrograde aortic root injection, dilated left main (LCA) and circumflex (CX) vessels with the fistulous connection to the right ventricle (arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
- Reliable, complete, noninvasive 3-dimensional imaging of the coronary vasculature is advantageous. Traditionally, MRI has been a good alternative for imaging proximal coronary abnormalities, and newer imaging sequences have provided improved anatomic imaging as well as indices of coronary flow and function. Spatial resolution is often limiting, and the distal course and insertion of the fistulous connection may not be well imaged.
- Recently, multidetector row computed tomography (MDCT) cardiac imaging has provided excellent distal coronary artery and side branch imaging. Imaging of an entire 3-dimensional volume and the heart can be acquired within 20 seconds, with better temporal and spatial resolution than MR. Several authors now advocate consideration of MDCT in imaging of coronary anomalies (see Media file 3).6
Three-dimensional multidetector row computed tomographic image showing a circumflex artery fistula. The left main stem is greatly dilated (arrow) and a dilated, tortuous circumflex artery becomes aneurysmal (An) before draining into the coronary sinus. Note also the left anterior descending (LAD) branches arising from this dilated vessel (arrowhead). (Image courtesy of Manghat NE, Morgan-Hughes GJ, Marshall AJ, Roobottom CA. Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart. Dec 2005;91(12):1515-22.)
- Stress thallium studies may be used to document areas of myocardial ischemia before and after operative repair.
Other Tests
- ECG may demonstrate the effects of volume load on the left ventricle and left atrium. Rarely, in the presence of coronary steal, ischemic changes and/or arrhythmias may be evident.
- Chest radiograph findings are generally normal, except in the presence of significant shunt flow, at which time cardiomegaly may be evident.
More on Coronary Artery Fistula |
| Overview: Coronary Artery Fistula |
 Differential Diagnoses & Workup: Coronary Artery Fistula |
| Treatment & Medication: Coronary Artery Fistula |
| Follow-up: Coronary Artery Fistula |
| Multimedia: Coronary Artery Fistula |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
Padfield GJ. A case of coronary cameral fistula. Eur J Echocardiogr. May 4 2009;[Medline].
Cemri M, Sahinarslan A, Akinci S, Arslan U. Dual coronary artery-pulmonary artery fistulas. Can J Cardiol. Mar 2009;25(3):e95. [Medline].
Schamroth C. Coronary artery fistula. J Am Coll Cardiol. Feb 10 2009;53(6):523. [Medline].
Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation. May 1979;59(5):849-54. [Medline].
Weymann A, Lembcke A, Konertz WF. Right coronary artery to superior vena cava fistula: imaging with cardiac catheterization, 320-detector row computed tomography, magnetic resonance imaging, and transesophageal echocardiography. Eur Heart J. May 20 2009;[Medline].
Chen ML, Lo HS, Su HY, Chao IM. Coronary artery fistula: assessment with multidetector computed tomography and stress myocardial single photon emission computed tomography. Clin Nucl Med. Feb 2009;34(2):96-8. [Medline].
Saglam H, Koçogullari CU, Kaya E, Emmiler M. Congenital coronary artery fistula as a cause of angina pectoris. Turk Kardiyol Dern Ars. Dec 2008;36(8):552-4. [Medline].
Ma ES, Yang ZG, Guo YK, Zhang XC, Sun JY, Wang RR. [Clinical value of 64-slice CT angiography in detecting coronary artery anomalies]. Sichuan Da Xue Xue Bao Yi Xue Ban. Nov 2008;39(6):996-9. [Medline].
[Guideline] Society of Thoracic Surgeons Workforce on Evidence Based Surgery. Antibiotic prophylaxis in cardiac surgery. Part 1, duration of prophylaxis. 2005;[Full Text].
Armsby LR, Keane JF, Sherwood MC, et al. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol. Mar 20 2002;39(6):1026-32. [Medline].
Carrel T, Tkebuchava T, Jenni R, et al. Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results. Cardiology. Jul-Aug 1996;87(4):325-30. [Medline].
Culham JAG. Abnormalities of the coronary arteries. In: Freedom RM, Mawson JB, Yoo SJ, eds. Congenital Heart Disease: Textbook of Angiocardiography. Armonk, NY: Futura Publishing; 1997:849-67.
De Wolf D, Vercruysse T, Suys B, et al. Major coronary anomalies in childhood. Eur J Pediatr. Dec 2002;161(12):637-42. [Medline].
Demirkilic U, Gunay C, Bolcal C, et al. Are discrete coronary artery fistulae different from coronary arteriovenous malformations?. J Card Surg. Mar-Apr 2005;20(2):124-8. [Medline].
Farooki ZQ, Nowlen T, Hakimi M, Pinsky WW. Congenital coronary artery fistulae: a review of 18 cases with special emphasis on spontaneous closure. Pediatr Cardiol. Oct 1993;14(4):208-13. [Medline].
Freedom RM, Benson LN. The etiology of myocardial ischemia: surgical considerations. In: Pulmonary Atresia with Intact Ventricular Septum. Armonk, NY: Futura Publishing Co; 1989:233.
Gittenberger-de Groot AC, Sauer U, Bindl L, et al. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol. Feb 1988;18(2):243-58. [Medline].
Latson LA, Forbes TJ, Cheatham JP. Transcatheter coil embolization of a fistula from the posterior descending coronary artery to the right ventricle in a two-year-old child. Am Heart J. Dec 1992;124(6):1624-6. [Medline].
Mahoney LT, Schieken RM, Lauer RM. Spontaneous closure of a coronary artery fistula in childhood. Pediatr Cardiol. 1982;2(4):311-2. [Medline].
Manghat NE, Morgan-Hughes GJ, Marshall AJ, Roobottom CA. Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart. Dec 2005;91(12):1515-22. [Medline].
McMahon CJ, Nihill MR, Kovalchin JP, et al. Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion. Tex Heart Inst J. 2001;28(1):21-5. [Medline]. [Full Text].
Moskowitz WB, Newkumet KM, Albrecht GT, et al. Case of steel versus steal: coil embolization of congenital coronary arteriovenous fistula. Am Heart J. Mar 1991;121(3 Pt 1):909-11. [Medline].
Parga JR, Ikari NM, Bustamante LN, et al. Case report: MRI evaluation of congenital coronary artery fistulae. Br J Radiol. Jun 2004;77(918):508-11. [Medline].
Reidy JF, Tynan MJ, Qureshi S. Embolisation of a complex coronary arteriovenous fistula in a 6 year old child: the need for specialised embolisation techniques. Br Heart J. Apr 1990;63(4):246-8. [Medline].
Said SA, el Gamal MI, van der Werf T. Coronary arteriovenous fistulas: collective review and management of six new cases--changing etiology, presentation, and treatment strategy. Clin Cardiol. Sep 1997;20(9):748-52. [Medline].
Tkebuchava T, Von Segesser LK, Vogt PR, et al. Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results. J Cardiovasc Surg (Torino). Feb 1996;37(1):29-34. [Medline].
Trehan V, Yusuf J, Mukhopadhyay S, et al. Transcatheter closure of coronary artery fistulas. Indian Heart J. Mar-Apr 2004;56(2):132-9. [Medline].
Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg. Mar 1983;35(3):300-7. [Medline].
Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. Jun 1995;35(2):116-20. [Medline].
Further Reading
- Relevant clinical guidelines include the following:
- American College of Cardiology (ACC) and American Heart Association (AHA) 2005 guidelines for the management of patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal aortic): a collaborative report from the American Association for Vascular Surgery/Society for Vascular Surgery, Society for Cardiovascular Angiography and Interventions, Society for Vascular Medicine and Biology, Society of Interventional Radiology, and the ACC/AHA Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Peripheral Arterial Disease)
- American College of Radiology Appropriateness Criteria for suspected congenital heart disease in the adult
- Relevant clinical trials include the following:
- Related eMedicine topics include the following:
Keywords
coronary artery fistula, CAF, coronary cameral fistula, coronary arteriovenous fistula, heart disease, coronary fistula, cardiac anomalies, cardiac fistula, cardiac disease, coronary artery anomaly, coronary arterial-venous fistula, CAVF, coronary-pulmonary artery fistula, congestive heart failure, CHF, pulmonary artery branch stenosis, coarctation of the aorta, pulmonary stenosis, coronary stenosis, aortic atresia, myocardial infarction, arrhythmias, infectious endocarditis, aneurysm, treatment, diagnosis
